One in a Million ®

NORD raises awareness of rare diseases by telling the stories of affected individuals and families. In these “One in a Million” stories, we hope to communicate that every life is important and no disease is rare when it affects someone you love.

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Ian Young

There are so many descriptions that could apply to Ian Young—proud Canadian, humorist, radio talk-show interviewer, public speaker, movie buff, gourmet cook,avid concert-goer, dollar-store enthusiast. “Victim” is not a phrase that Ian would ever use about himself, despite having developed a rare, life-altering neurological disorder. As he will readily tell you, “I am a survivor-Never say never!”

In early spring 2004, Ian was a healthy, athletic, and active 40-year-old, thoroughly enjoying his work in one of Calgary, Canada’s finest hotels. A mild sensation of “feeling different” one day, however, marked the quiet beginning of what was to become a nightmarish fight for survival.

Ian sought care at a local walk-in clinic, one of several funded through Canada’s government-sponsored healthcare system. From there, eventually, he was referred to a local hospital but it was several days before a bed became available for him. Meanwhile, his symptoms became more pronounced with each passing day. What had started as a vaguely unwell feeling had progressed to overwhelming weakness and balance problems.

While it was obvious that a powerful neurological assault had taken place, Ian’s physicians were baffled by the rapidly worsening cascade of events. His left side became paralyzed and, eventually, he also lost his ability to speak or swallow. Finally, a succession of spinal taps, MRIs, scans, and blood tests yielded a diagnosis—Ian had developed a rare neurological disorder called progressive multifocal leukoencephalopathy (PML)

PML is caused by the destruction of myelin, a fatty substance that coats, protects, and insulates nerve fibers in the brain and spinal cord. These bundles of myelin-sheathed nerves are referred to as the “white matter” of the brain. In PML, the destruction of myelin is caused by the re-activation of a common virus, the “JC virus”.

The “JC” virus is usually harmless, causing no symptoms. It gains entrance to most people’s systems during childhood, and it is estimated that anywhere from 85-90% of the healthy adult population harbors the JC virus in their system. It does not cause any symptoms or damage if the immune system is normal.

During a period of immune suppression, however, the harmless nature of the JC virus can change. For example, during chemotherapy for cancer, or while receiving medication for organ transplants or autoimmune disorders, or as a result of other severe, immune-compromising disorders and/or viruses, a once strong immune system can come under assault. The JC virus then replicates and multiplies, becoming an “opportunistic” infection. It ultimately enters the bloodstream and makes its way to the brain, targeting and destroying the cells that produce myelin. It is typical for the symptoms to begin quietly and insidiously, as in Ian’s case, and then to become rapidly overwhelming. For Ian, blood tests revealed that a virus had severely compromised his immune system.

Presently, there is no treatment for PML. Sometimes, stopping medication that has compromised the immune system may be successful in staunching the viral onslaught. In the case of an HIV infection, beginning highly active anti-retroviral medication (HAART) can often, but not always, restore the immune system sufficiently to fight the JC virus. For many, however, there is simply no medical means to restore the immune system to proper functioning. PML is often fatal, with victims living for only a few months. Rarely, people do survive due to a spontaneous improvement of their immune function.

Fortunately, Ian had a “diehard advocate” on his side¾his sister Yvette. Living over 300 miles away in the province of Alberta, married with young children and a full-time job, Yvette became Ian’s legal guardian and spokesperson. She drove out to Calgary every weekend and stayed at her brother’s bedside, providing care for him and relentlessly advocating on his behalf.

Each time she would visit Ian, Yvette observed increasing deterioration and weight loss. In fact, Ian’s weight had drastically plunged, since he had became unable to swallow either food or fluids. His weight went as low as 87 pounds, indicating severe malnutrition in a man who is 5’8” tall. “So…my routine was to visit with Ian on weekends, see doctors when I could (which wasn’t often, because it was the weekend), and phone the doctors during the week,” says Yvette. It was through relentless and persistent phone calls that Yvette convinced the doctors that her brother was starving to death and desperately needed a feeding tube. Her efforts finally paid off, and a feeding tube was placed.

She also worked to get Ian transferred to the University of Alberta Medical Center in Edmonton, Alberta, closer to where she lived. As Ian describes it, “I arrived at the University of Alberta, diaper-clad and in a Christopher Reeve-type wheelchair—I couldn’t even support my neck.”

Yvette visited Ian every day, often bringing along her then 6-year-old son, Scott. “There were several times that it seemed to me that Ian was giving up on himself,” says Yvette--“that he wanted to die. I spoke with him many times about how much we loved him and that it was important to have the will to live.”

For the first several months that he was in Edmonton, Ian languished in a dementia ward in a long-term care facility, where most of the other patients were in the 80s or 90s. Finally, he was accepted into the Glenrose Rehabilitation Hospital’s Acquired Brain Injury Program in Edmonton. The hospital had no experience with PML and was reluctant to work with Ian. But, “they took a gamble”, he says, and it was a gamble that would benefit both him and his doctors.

Within one week of arrival at the rehab unit, Ian was able to swallow. Dr. Juliana Nagy, an acquired brain injury specialist, also weaned him off the laxatives and antidepressants that he had been receiving while on the long-term dementia ward. His condition began slowly to improve.

Several meetings with a Glenrose psychologist revealed that Ian had one more diagnosis¾post-traumatic stress disorder (PTSD), possibly brought on by his experiences since the onset of his disease. With the guidance of Dr. Lynda Phillips, Ian was able to learn mental exercises to help him cope with the PTSD.

Midway into his rehabilitation program, Ian was able to “graduate” to an independent living suite in the hospital. This was a modified apartment set-up in which “they took me out shopping and I had to make my own meals, do my own cleaning, wake myself up, and keep all my therapy appointments.”

To the sheer amazement of the Glenrose staff, it became evident towards the end of his rehabilitation program that Ian would be able to live independently again. “Forty-eight days after being admitted to Glenholm, I left talking, laughing, eating a granola bar, diaperless and clean, with my weight up to 150 pounds,” he says.

Ian currently lives entirely on his own in an apartment that is rent-subsidized by the Canadian government, and receives disability income. He requires a 4-wheel walker when going outdoors and a cane indoors, and has learned to navigate the public transportation system to get around his beloved city of Edmonton. He has a volunteer position as a media correspondent at a local radio station sponsored by the University of Alberta, CJSR. Ian not only conducts interviews for the station, but has also learned the art of editing and formatting. In addition, “I am registered with every government agency to find employment, but have not had much luck yet.”

When asked just how he coped with such a devastating disorder and the added misery of being left to languish in a ward, Ian states unequivocally that it was the love and support of his family, and in particular, his sister Yvette. “She never took ‘no’ for an answer.”

Ian also maintains close contact with the Glenrose staff, and has done public speaking at various brain injury forums. He is the first brain injury survivor in Canada to sit on the provincial Brain Injury Interagency Committee, and is headed to Montreal this summer to speak and participate in a symposium sponsored by this Committee.

Ian also maintains a relationship with a small but solid online PML support group—
http://health.groups.yahoo.com/group/PMLSurvivors/ . He refers to his fellow PML sufferers as “comrades” who are “fighting the good fight.” He regularly posts comments there and lends support to others who are fighting this devastating disorder.

Yvette, Ian’s stalwart and dedicated sister, knows that her persistence probably made the difference between life and death for him. But she also believes that “Ian can take full credit for working hard at his recovery and making the tremendous progress that he has.”